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PubMed1995

Effects of oxygen radicals on ciliary motility in cultured human respiratory epithelial cells.

There are few reports about direct effects of specific oxygen products on ciliary function because of their instability and reactivity. We investigated the direct effects of superoxide anion (O2-) and of hydrogen peroxide (H2O2) on the ciliary function of human respiratory epithelial cells, using monolayer cell cultures, high speed video analysis of frequency (CBF), amplitude (CBA), and coordination of ciliary beats and evaluating the surface structural changes of ciliated cells at the same time. 10(-2) M H2O2 decreased ciliary beat activity. The CBF was 36.5 +/- 4.4% and the CBA was 51.0 +/- 3.8% of the baseline (time = 0) after 5 min (all p < 0.001). Catalase (2 micrograms/ml) abolished the ciliotoxic effect of H2O2. The O2- produced by reaction of xanthine (0.06 mM)-xanthine oxidase (0.04 U/ml) caused a temporary rapid increase of 26.8 +/- 1.7% in CBF and an increase of 42.5 +/- 4.1% in CBA after 15 sec (all p < 0.001). Superoxide dismutase significantly reduced these increases. Results indicated that O2- activated ciliary function with a temporary increase in O2(-)-production. This suggests that the removal of H2O2 from the O2- reaction is important in improving mucociliary clearance in excessive oxygen metabolites.

Cells, Cultured↗
PubMed1999

Effects of free radicals on ciliary movement in the human nasal epithelial cells.

OBJECTIVE: There have been few reports on the effects of free oxygen radicals on ciliary mobility of nasal respiratory epithelial cells. The aim of this study was to determine the effects of free radicals and antioxidants on human nasal epithelial cells (HNECs) using video-computerized analysis. METHODS: Human nasal epithelial cells were obtained from the nasal cavity of normal volunteers. Ciliary beat frequency (CBF) was calculated as the mean value of ten randomly selected cells. The proportion of the area with normal CBF (above 8 Hz) was calculated from 10 randomly selected sites per specimen. Free radicals were produced by xanthine-xanthine oxidase enzymatic system. The generation of free radicals was confirmed by chemoilluminometer. CBF and the proportion of the area with normal CBF were measured at every 5 min for 30 min after the addition of enzyme. For the evaluation of the antioxidant effects on free radical-mediated ciliary slowing in HNECs, cells were incubated in superoxide dismutase solution (300 unit/ml) for 30 min and 3-aminobenzamide (5 mM). RESULTS: Superoxide produced by 0.4 mM xanthine and 400 miliunit/ml xanthine oxidase decreased CBF (7.71 +/- 1.91 Hz). A total of 2 min later, ciliary slowing was evident (3.87 +/- 1.10 Hz). Regarding the changes in proportion of epithelial area that showed normal CBF experimental group showed a significant decrease in percentage of epithelial area with normal CBF over time. Superoxide dismutase prevented ciliary slowing (8.76 +/- 0.99 Hz). Moreover, 3-aminobenzamide, an inhibitor of the DNA repair enzyme poly-ADP ribose polymerase, prevented inhibition of CBF (8.32 +/- 0.61 Hz). CONCLUSIONS: These results suggest that oxygen-mediated damage to DNA may be the mechanism of the deterioration effects of oxygen radicals on the ciliated respiratory nasal epithelium.

Antioxidants↗
PubMed2004

[Molecular basis of the primary ciliary dyskinesias].

INTRODUCTION: The primary ciliary dyskinesias (PCD) are rare diseases characterised by infection of the airways due to impaired muco-ciliary clearance. Half the patients have situs inversus making up Kartagener's syndrome. STATE OF THE ART: Primary cilia play a role in development. In the adult ciliated cells occur mainly in the airways and the genital tract. The axoneme, the internal structure of the cilia, is made up of a central pair of microtubules surrounded by peripheral doublets carrying the inner and outer dynein arms. These multiprotein complexes are composed of chains of dynein whose ATPase activity is the basis of ciliary movement. Structural and functional abnormalities of the respiratory ciliated cells are the cause of PCD, diseases that are heterogeneous at both the genetic and ultrastructural levels. PERSPECTIVES: There are more than two hundred axonemal proteins. The synthesis and assembly of these proteins are controlled by transcription factors and intraflagellar transport molecules respectively. The genes that code for these proteins are as numerous as candidate genes for PCD. CONCLUSIONS: To date only two dynein genes, DNA11 and DNAH5, have been implicated and only in individuals suffering from PCD with absence of outer dynein arms.

Animals↗
PubMed2004

Pregnancy after ICSI with ejaculated immotile spermatozoa from a patient with immotile cilia syndrome: a case report and review of the literature.

This study presents a case of intracytoplasmic sperm injection (ICSI) with ejaculated immotile spermatozoa from a patient with immotile cilia syndrome. Semen analysis of the patient suffering from immotile cilia syndrome revealed an extreme oligoasthenoteratozoospermia (OAT: count <1.4 x 10(6)/ml, 0% motility and 3% normal morphology). Electron microscopy of sperm flagella showed the absence of inner and outer dynein arms. During the ICSI cycle, the hypo-osmotic swelling test (HOS) was used for the identification of viable spermatozoa in the pool of immotile spermatozoa for ICSI. A normal fertilization rate was found in eight out of the 12 oocytes. A first fresh double embryo transfer resulted in a late miscarriage at 21 weeks. A second healthy singleton pregnancy occurred after transfer of two frozen-thawed embryos from the same ICSI procedure. Although only one successful ICSI case of the immotile cilia syndrome combined with HOS is described here, HOS might be a simple but valuable tool to obtain normal fertilization and pregnancy for patients suffering from immotile spermatozoa.

Adult↗
PubMed1988

Young's syndrome (a case report).

A case of Young's syndrome is described presenting with obstructive azoospermia, sinusitis, otitis media and bronchitis. It is essential to examine ciliary function and ultrastructure in order to make this diagnosis.

Adult↗
PubMed1999

Regulation of mucociliary clearance in health and disease.

Airway secretions are cleared by mucociliary clearance (MCC), in addition to other mechanisms such as cough, peristalsis, two-phase gas-liquid flow and alveolar clearance. MCC comprises the cephalad movement of mucus caused by the cilia lining the conducting airways until it can be swallowed or expectorated. MCC is a very complex process in which many variables are involved, all of which may modify the final outcome. The structure, number, movement and co-ordination of the cilia present in the airways as well as the amount, composition and rheological properties of the periciliary and mucus layers are determinants of MCC. Physiological factors such as age, sex, posture, sleep and exercise are reported to influence MCC due to a change in the cilia, the mucus or the periciliary layer, or a combination of these. Environmental pollution is suspected to have a depressant effect on MCC dependent on different factors such as pollutant concentration and the duration of exposure. Most studies focus on sulphur dioxide, sulphuric acid, nitrogen dioxide and ozone. Tobacco smoke and hairspray have been noted to have a negative influence on MCC. Some diseases are known to affect MCC, mostly negatively. The underlying mechanism differs from one illness to another. Immotile cilia syndrome, asthma, bronchiectasis, chronic bronchitis, cystic fibrosis and some acute respiratory tract infections are among the most frequently reported. The present paper reviews normal mucociliary clearance and the effects of diseases on this process.

Ciliary Motility Disorders↗
PubMed1999

Effects of drugs on mucus clearance.

Mucociliary clearance (MCC), the process in which airway mucus together with substances trapped within are moved out of the lungs, is an important defence mechanism of the human body. Drugs may alter this process, such that it is necessary to know the effect of the drugs on MCC. Indeed, agents stimulating MCC may be used therapeutically in respiratory medicine, especially in patients suspected of having an impairment of their mucociliary transport system. In contrast, caution should be taken with drugs depressing MCC as an undesired side-effect, independently of their therapeutic indication. Since cough clearance (CC) serves as a back-up system when MCC fails, the influence of drugs must be examined not only on MCC but also on CC. Ultimately, the clinical repercussions of alterations in mucus transport induced by drug administration must be studied. Tertiary ammonium compounds (anticholinergics), aspirin, anaesthetic agents and benzodiazepines have been shown to be capable of depressing the mucociliary transport system. Cholinergics, methylxanthines, sodium cromoglycate, hypertonic saline, saline as well as water aerosol have been shown to increase MCC. Adrenergic antagonists, guaifenesin, S-carboxymethylcysteine, sodium 2-mercapto-ethane sulphonate and frusemide have been reported not to alter the mucociliary transport significantly. Amiloride, uridine 5'-triphosphate (UTP), quaternary ammonium compounds (anticholinergics), adrenergic agonists, corticosteroids, recombinant human deoxyribonuclease (rhDNase), N-acetylcysteine, bromhexine and ambroxol have been reported either not to change or to augment MCC. Indirect data suggest that surfactant as well as antibiotics may improve the mucociliary transport system. As for the influence of drugs on CC, amiloride and rhDNase have been demonstrated to increase the effectiveness of cough. A trend towards an improved CC was noted after treatment with adrenergic agonists. The anticholinergic agent ipratropium bromide, which is a quaternary ammonium compound, has been suggested to decrease CC significantly. Bromhexine, ambroxol and neutral saline seemed not to alter CC, either positively or negatively. Finally, treatment with either amiloride, recombinant human deoxyribonuclease, bromhexine, ambroxol, N-acetylcysteine, S-carboxymethylcysteine or hypertonic saline has been suggested as a possible cause of clinical improvement in patients, such as the experience of dyspnoea, the case of expectoration or the frequency of infective exacerbations. Other agents did not show a clinical benefit.

Animals↗
PubMed2005

The centrosome in human genetic disease.

The centrosome is an indispensable component of the cell-cycle machinery of eukaryotic cells, and the perturbation of core centrosomal or centrosome-associated proteins is linked to cell-cycle misregulation and cancer. Recent work has expanded our understanding of the functional complexity and importance of this organelle. The centrosomal localization of proteins that are involved in human genetic disease, and the identification of novel centrosome-associated proteins, has shown that numerous, seemingly unrelated, cellular processes can be perturbed by centrosomal dysfunction. Here, we review the mechanistic relationship between human disease phenotypes and the function of the centrosome, and describe some of the newly-appreciated functions of this organelle in animal cells.

Animals↗
PubMed2001

Respiratory ciliary function in bone marrow recipients.

Bone marrow transplantation (BMT) recipients, particularly those with chronic graft-versus-host disease (GVHD), suffer from respiratory tract problems, including bronchiolitis obliterans (BO) and recurrent lower respiratory tract infections. Minute cilia beat continuously on the surface of respiratory mucosa, and this beating maintains the sterility of the lower respiratory tract. Dysfunction of respiratory cilia could lead to development of recurrent respiratory tract infections, which are also features of BMT recipients, although ciliary function has not been systematically studied among these subjects. We have, therefore, investigated the ciliary beat frequency (CBF) of 36 Chinese patients who had undergone allogeneic BMT. The CBF was significantly lower in the BMT group compared to controls (P < 0.001). The reduction in CBF was more severe in patients with cGVHD and BO compared with their counterparts (P = 0.048 and P = 0.077, respectively). There was a correlation between CBF with forced expiratory flow rate FEF (P = 0.024) and forced expiratory volume FEV (P = 0.044). We conclude that abnormal ciliary clearance is a common feature after allogeneic BMT, particularly among patients with BO and cGVHD. Further studies are indicated to evaluate this important phenomenon, which could be an important cause of the susceptibility for BMT recipients to respiratory infections.

Adolescent↗
PubMed2006

A prospective study of respiratory ciliary structure and function after stem cell transplantation.

We prospectively investigated the morphological and ciliary function abnormalities in 19 consecutive Chinese patients undergoing hemopoietic stem cell transplantation (HSCT) and studied their relationship with pulmonary complications. The percentage of structural ciliary abnormalities preceding HSCT was comparable to normal controls, but increased up to 1-year post-HSCT. However, the abnormalities did not correlate with ciliary or pulmonary function. Ciliary beat frequency (CBF) for patients undergoing autologous and allogeneic SCT was lower than that of matched controls, with a further decline at one year. There was, however, no significant change in pulmonary function for the whole cohort. There was considerable variation in CBF and ciliary abnormalities in all cases during 3-month interval assessments. Regular ciliary assessment did not predict the only two patients who eventually suffered from bronchiolitis obliterans (BO). We conclude that structural and functional ciliary abnormalities are common in recipients of HSCT, and predict post-HSCT deterioration. However, there is no evidence to show that CBF monitoring may be of prospective benefit.

Adult↗
PubMed2000

Plasma membrane polarity of polymorphonuclear leucocytes from children with primary ciliary dyskinesia.

BACKGROUND: Polymorphonuclear leucocytes (PMN) from subjects with primary ciliary dyskinesia (PCD) can have abnormal locomotory systems. The locomotory activity of PMN is the result of biochemical events mediated by the plasma membrane. In this study we investigated plasma membrane polarity of PMN from children with PCD. DESIGN: Membrane polarity was studied in 11 children with PCD and in healthy controls by measuring the steady-state fluorescence excitation and emission spectra of 2-dimethylamino[6-lauroyl]naphthalene (Laurdan), which is known to be incorporated at the hydrophobic-hydrophilic interface of the bilayer, displaying spectral sensitivity to the polarity of its surroundings. Laurdan shows a marked steady-state emission red shift in polar solvents, with respect to nonpolar solvents. Moreover, the effect of the microtubule disassembling agent colchicine on PMN membrane polarity was evaluated. RESULT: Our results show a red shift of the fluorescence excitation and emission spectra of Laurdan in PMN from the PCD group with respect to the control group. These data indicate an increase in membrane polarity of PMN from the PCD group. Treatment of PMN with colchicine induced a red shift in the Laurdan excitation and emission spectra with the same trend observed in PMN from the PCD group. CONCLUSION: PMN from children with PCD are characterized by an increased plasma membrane polarity. These changes could be the basis of the modifications in the locomotory activities of PMN. The observed alterations may be attributed to abnormalities in the cytoskeleton.

2-Naphthylamine↗
PubMed2000

Ciliary assessment in bronchiectasis.

Bronchiectasis is a common condition among the Oriental population and affected patients suffer from chronic sputum production punctuated by recurrent infective exacerbations. Cilia are minute structures present on the surface of respiratory and other epithelial cells that beat continuously to maintain a sterile mucosal surface in the respiratory tract. Patients with primary ciliary dyskinesia could potentially develop recurrent sinotrachrobronchitis, bronchiectasis, serous otitis media, hydrocephalus, and male infertility. The assessment of cilia has, however, received little attention until recently and generally involves elaborate methods that require complex and expensive technology. This brief article discusses application of the saccharine test, light microscopy assessment of ciliary beat, and transmission electron microscopy assessment of the ultrastructure of cilia. The rationale and indications for ciliary assessment are also listed along with illustrations showing ciliary structure, equipment required for sampling and assessment of cilia, and transmission electron micrographs of ciliary ultrastructural abnormalities.

Bronchiectasis↗
PubMed2001

Genetic contributions to rare childhood lung diseases.

Epidemiological studies have suggested that many rare diseases with respiratory involvement have a genetic component. Molecular advances have increased the understanding of the pathophysiology of these diseases which has led to better diagnostic and prognostic methods. There may be many genes responsible for diseases such as primary ciliary dyskinesia and systemic lupus erythematosus in addition to the effect of modifier genes. The genotype:phenotype correlation in these diseases remains to be elucidated. In some diseases, such as familial dysautonomia and sickle cell, the gene has been identified which allows for accurate pre-natal testing. Further, in diseases where the genetic abnormality is known, such as chronic granulomatous disease, gene therapy remains a realistic prospect and phase I studies are about to commence or currently underway. This article reviews those rare diseases in which there is or is likely to be a significant genetic contribution.

Anemia, Sickle Cell↗
PubMed2000

Elevated levels of macrophage-stimulating protein in induced sputum of patients with bronchiectasis.

We recently showed that macrophage-stimulating protein (MSP), a serum protein homologous to hepatocyte growth factor, promotes ciliary motility by activating its receptor, RON, on the airway ciliated epithelium. To investigate the functional involvement of MSP and RON in bronchiectasis, in which mucociliary clearance (MCC) is impaired, first we examined RON expression on the bronchial ciliated epithelium of patients with bronchiectasis. We confirmed RON expression at the apical surface of bronchial ciliated epithelium of patients with bronchiectasis as well as those of normal human bronchus. Next, we examined whether MSP is present in sputum of patients with bronchiectasis and normal control subjects. By Western blotting, we found that half of the MSP in sputum is present as a biologically active alpha/beta chain heterodimer (mature MSP). In addition, we found that the MSP concentrations in sputum were significantly elevated in patients with bronchiectasis (n=8; 16.8+/-3.0 ng ml(-1)) compared with normal controls (n=9; 8.4+/-2.4 ng ml(-1); P<0.05). In contrast, the difference in concentrations of serum MSP (pro-MSP) was not significant between the two groups. These results indicate that (i) MSP is supplied to the airways and converted to a biologically active form, (ii) MSP is increased in patients with bronchiectasis compared with normal controls. Taken together, our findings suggest that increased MSP may be involved in compensation for impaired MCC in bronchiectasis.

Adult↗